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[伴有凹陷性水肿的缓解型血清阴性对称性滑膜炎。1例报告]

[Remitting seronegative symetrical synovitis with pitting edema. One case report].

作者信息

Pimentel-León Rafael Rubén, García-Chávez Margarita, Chávez-Sánchez Iliana Nelly

机构信息

Instituto Mexicano del Seguro Social, Hospital General de Zona No. 1, Servicio de Reumatología. La Loma, Tlaxcala, México.

Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado, Hospital Regional Ignacio Zaragoza, Servicio de Inmunología y Alergia. Ciudad de México, México.

出版信息

Rev Med Inst Mex Seguro Soc. 2023 May 2;61(3):363-369.

PMID:37216683
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10437230/
Abstract

BACKGROUND

RS3PE (remitting seronegative symmetrical synovitis with edema and pitting) is a rare entity of unknown etiology that has been related to genetic predisposition due to the presence of HLA-A2 in 50% of cases and less frequently HLA-B7. Its pathogenesis is unknown, but it has been related to growth factors, and some mediators (TNF, IL-6). It is common in elderly people and the course of this illness presents with acute symmetrical polyarthritis, accompanied by edema in hands and feet. The diagnosis requires a high index of suspicion and to differentiate it from other entities such as rheumatoid arthritis, complex regional pain syndrome, rheumatic polymyalgia, in addition to ruling out malignant neoplasms, since there are many reports of its association with both solid and hematological neoplasms, being of bad prognosis when there is association. When there is no association with cancer, it responds well to the use of low doses of steroids and its prognosis is usually favorable.

CLINICAL CASE

80-year-old woman with an acute onset with polyarthralgia, functional limitation associated with pitting edema in hands and feet. After approaching the patient and ruling out associated neoplasms, it was diagnosed RS3PE. It was managed with prednisone, observing a good response, with remission of the manifestations at 6 weeks and subsequent suspension of the steroid.

CONCLUSIONS

RS3PE is a rare entity, and a high index of suspicion is required for the diagnosis. A complete approach is important to rule out cancer in patients affected with this syndrome. Prednisone continues to be the best therapeutic option.

摘要

背景

缓解性血清阴性对称性滑膜炎伴水肿和凹陷(RS3PE)是一种病因不明的罕见病症,50%的病例与基因易感性有关,较少与HLA - B7相关。其发病机制尚不清楚,但与生长因子及一些介质(肿瘤坏死因子、白细胞介素 - 6)有关。多见于老年人,病程表现为急性对称性多关节炎,伴有手足水肿。诊断需要高度怀疑,并与类风湿关节炎、复杂性区域疼痛综合征、风湿性多肌痛等其他病症相鉴别,此外还要排除恶性肿瘤,因为有许多关于其与实体肿瘤和血液系统肿瘤相关的报道,若存在关联则预后不良。当与癌症无关联时,使用低剂量类固醇治疗效果良好,预后通常较好。

临床病例

一名80岁女性,急性起病,有关节痛、功能受限,伴有手足凹陷性水肿。对患者进行检查并排除相关肿瘤后,诊断为RS3PE。采用泼尼松治疗,观察到良好反应,6周时症状缓解,随后停用类固醇。

结论

RS3PE是一种罕见病症,诊断需要高度怀疑。全面检查对于排除该综合征患者的癌症很重要。泼尼松仍然是最佳治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a426/10437230/510a9e91d298/04435117-61-3-363-c002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a426/10437230/1a34a4c00e4d/04435117-61-3-363-f001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a426/10437230/08bae44c44ef/04435117-61-3-363-c001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a426/10437230/510a9e91d298/04435117-61-3-363-c002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a426/10437230/1a34a4c00e4d/04435117-61-3-363-f001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a426/10437230/08bae44c44ef/04435117-61-3-363-c001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/a426/10437230/510a9e91d298/04435117-61-3-363-c002.jpg

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